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| == Diagnostic Study of Choice == | | == Diagnostic Study of Choice == |
| * Diagnosis of PNH is made by having peripheral blood cells which are deficient in glycophosphatidylinositol (GPI)-linked proteins.
| | === Minimal essential diagnostic criteria === |
| * The diagniosis is also clinically and there are three types of PNH
| | * Diagnosis of paroxysmal nocturnal hemoglobinuria is based on the minimal essential diagnostic criteria. The main diagnostic test for this criteria is [[flow cytometry]]. |
| We also distinguish between clinically apparent and subclinical PNH, and between "classical" PNH versus PNH that occurs in the setting of an underlying bone marrow disorder. The International PNH Interest Group (I-PIG) has proposed a working diagnostic classification that includes the following three categories [9]:
| | * The flow cytometry approach is used to reveal the deficiency of [[GPI anchor]] on the [[RBCs]]. |
| | | * Other measures used in the diagnostic criteria include the folllowing: |
| ●'''Classic PNH''' – PNH with clinical evidence of intravascular hemolysis, without another bone marrow abnormality.
| | ** Assessment of the [[hemolytic]] parameters |
| | | ** [[Bone marrow]] examination |
| ●'''PNH in the context of another bone marrow disorder''' – Evidence of hemolysis as well as another primary bone marrow abnormality such as aplastic anemia (AA), a myelodysplastic syndrome (MDS), or primary myelofibrosis (PMF).
| | * Supporting tests are included also in order to detect the PNH category. Below tables conclude both supporting tests and categories of PNH . |
| | | {| class="wikitable" |
| ●'''Subclinical PNH''' – A small population of PNH cells (ie, blood cells lacking GPI-anchored proteins), without clinical or laboratory evidence of hemolysis. This is more commonly detected in patients with another bone marrow disorder, although this association may reflect more extensive evaluation of this population rather than a truly greater incidence of subclinical PNH in individuals with other bone marrow disorders.
| | |+ |
| | | !Category of PNH |
| === Study of choice === | | !Characteristics |
| [Name of the investigation] is the gold standard test for the diagnosis of [disease name].
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| OR
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| The following result of [gold standard test] is confirmatory of [disease name]: | |
| * [Result 1]
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| * [Result 2]
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| OR
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| [Name of the investigation] must be performed when:
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| * The patient presents with [symptom/sign 1], [symptom/sign 2], and [symptom/sign 3].
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| * A [name of test] is positive for [sign 1], [sign 2], and [sign 3] in the patient. | |
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| OR
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| [Name of the investigation] is the gold standard test for the diagnosis of [disease name].
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| OR
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| The diagnostic study of choice for [disease name] is [name of the investigation].
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| OR
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| There is no single diagnostic study of choice for the diagnosis of [disease name].
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| OR
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| There is no single diagnostic study of choice for the diagnosis of [disease name], but [disease name] can be diagnosed based on [name of the investigation 1] and [name of the investigation 2].
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| OR
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| [Disease name] is primarily diagnosed based on the clinical presentation. | |
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| OR
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| Investigations:
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| * Among the patients who present with clinical signs of [disease name], the [investigation name] is the most specific test for the diagnosis.
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| * Among the patients who present with clinical signs of [disease name], the [investigation name] is the most sensitive test for diagnosis. | |
| * Among the patients who present with clinical signs of [disease name], the [investigation name] is the most efficient test for diagnosis.
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| ==== The comparison of various diagnostic studies for [disease name] ====
| |
| {| | |
| |- style="background: #4479BA; color: #FFFFFF; text-align: center;"
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| ! style="background: #4479BA; color: #FFFFFF; text-align: center;" | Test
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| ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sensitivity | |
| ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Specificity | |
| |- | | |- |
| ! style="background: #696969; color: #FFFFFF; text-align: center;" |Test 1
| | |1- Classic PNH |
| | style="background: #DCDCDC; padding: 5px; text-align: center;" |...% | | | |
| | style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
| | * [[Hemolysis|Intravascular hemolysis]] evident |
| | * No another [[bone marrow]] disorder |
| | * Bone marrow morphology is normal with [[erythroid]] [[hyperplasia]] |
| | * No karyotype abnormality |
| |- | | |- |
| ! style="background: #696969; color: #FFFFFF; text-align: center;" |Test 2
| | |2- PNH in the setting of another specified bone marrow disorder |
| | style="background: #DCDCDC; padding: 5px; text-align: center;" |...% | | | |
| | style="background: #DCDCDC; padding: 5px; text-align: center;" |...% | | * Intravascular hemolysis evident |
| | * History of defined bone marrow abnormality |
| | * This category used to determine if the PNH is secondary to [[aplastic anemia]] or [[myelodysplastic syndrome]] |
| | * A [[chromosomal]] abnormality may be associated with myelodysplastic syndrome |
| | |- |
| | |3- Subclinical PNH |
| | | |
| | * No evidence of hemolysis |
| | * Patients with subclinical PNH are detected by the [[Flow cytometry|flow cytometric analysis]] |
| | * Mostly associated with bone marrow failure |
| |} | | |} |
| <small> [Name of test with higher sensitivity and specificity] is the preferred investigation based on the sensitivity and specificity</small>
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| ===== Diagnostic results =====
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| The following finding(s) on performing [investigation name] is(are) confirmatory for [disease name]:
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| * [Finding 1]
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| * [Finding 2]
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| ===== Sequence of Diagnostic Studies =====
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| The [name of investigation] must be performed when:
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| * The patient presented with symptoms/signs 1, 2, and 3 as the first step of diagnosis.
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| * A positive [test] is detected in the patient, to confirm the diagnosis.
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| OR
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| The various investigations must be performed in the following order:
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| * [Initial investigation]
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| * [2nd investigation]
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| === Name of Diagnostic Criteria ===
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| '''It is recommended that you include the criteria in a table. Make sure you always cite the source of the content and whether the table has been adapted from another source.'''
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| [Disease name] is primarily diagnosed based on clinical presentation. There are no established criteria for the diagnosis of [disease name].
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| OR
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| There is no single diagnostic study of choice for [disease name], though [disease name] may be diagnosed based on [name of criteria] established by [...].
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| OR
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| The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
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| OR
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| The diagnosis of [disease name] is based on the [criteria name] criteria, which includes [criterion 1], [criterion 2], and [criterion 3].
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| OR
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| [Disease name] may be diagnosed at any time if one or more of the following criteria are met:
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| * Criteria 1
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| * Criteria 2
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| * Criteria 3
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| OR
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| '''IF there are clear, established diagnostic criteria'''
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| The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
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| OR
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| The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
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| OR
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| The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
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| OR
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| '''IF there are no established diagnostic criteria'''
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| There are no established criteria for the diagnosis of [disease name].
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| ==References== | | ==References== |